Git Case Budd Chiari3.

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Git Case Budd Chiari3.

  1. 1. Case presentation: Dr. Mohammad Shaikhani. Dr. Mohammad Omer Mohammad. Sulaimanyah University. College of Medicine. Department of Medicine.
  2. 2. SH.K, 20 years old, Studen from Darbandixan,Sulaimanyiah, Iraq. Date of admission: 9.9.08 CC: abdominal distention for the last 2 weeks H.P.I: a female with abdominal distention and yellow discoloration of skin and sclera associated with pain during voiding and frequency with lower pelvic pain. There were no fever no rigor. P.M.H: not significant D.H: on HRT for irregular cycles. There is no Hx of blood transfusion, tattooing or drug injection “ addiction ”
  3. 3. Review of systems: Resp: SOB, no cough no sputum CVS: no palpitation, no leg edema. GIT: decr. Appetite, nausea, constipation. O/E: conscious oriented young lady Ting of jaundice, no cyanosis … mild pallor Chest: clear, N.V.B Precordium: NDR, no added sounds Abdomen: soft, hepatospleenomegally Ascitis with +ve shifting dullness and trans thrill Neurology: intact Vita signs …
  4. 4. Ix: LFT: Bilirubin 0.9 , 0.7 Alk. Phosphatase: 87 , 69 S. GOT: 8 , 26 S.GPT: 5 , 25 T.S.P: 7.6 , 7.7 PT: 21.7 sec , INR: 2.4 - 19.5 INR: 2.0 PCV: 42 , 37 CBP: Hb: 13.2 – MCV: 39.8 – MCH: 22.0 – MCHC: 33.3 – WBC: 4200 , 8700 – 80% N 17% L – PLT: 586.000
  5. 5. Bl. Film: RBC: normochromic normocytic screeninig for infection & infl. Disorders. WBC: Neutrophilia, Lymphopenia. Platelet: Markedly increased. ESR: 11 ,14 GUE … U/S: mild liver enlargement, coarse texture no focal lesion, mod-severe large spleen mod. Amount of ascitis.
  6. 6. Ascetic fluid aspiration: WBC = lymphocytosis Ptn= 4.5,4.8 Sugar= 100 AFB: negative Cytology= LC, no malignant cells ANA and AMC Ab Ads DNA Ab A LKM … -VE ANTILIVER KIDNEY MICROSOME= -VE Viral marker= -ve (twice) OGD= esophagitis, gastritis Peritoneal biopsy: no granuloma, no malig.
  7. 7. S. Copper: 150Mg Ceruloplasmin: 36.3mg RBS: 126 , RFT: Bl U: 11 , S Creat: 0.5 SGPT: 13 , SGOT: 18 , PT: 20.6 , INR: 2.1 Ascitic Fluid: Lymphocytosis: 70% , Pr: 5.2 Sug: 120 , no AFB CBP: Hb: 12.7 – MCV: 65.9 - MCH: 21.9 – MCHC: 33.3 – WBC: 13600 – PLT:813.000 Bl. Film: RBC: hypochromic microcytic anisopoikilocytosis elliptocytosis. WBC:mild Neutrophilia. Platelet: Markedly increased with anisothrombia many Giant cells seen.
  8. 8. S Pr. Elec: AlB: normal 58.20 Alpha 1: low 0.07 Alpha 2: low 3.65 Beta: low 6.62 Gama: high 31.46 Total: 8.20 gm/dl A/G : 1.39
  9. 9. Doppler study: 1 st : prominent caudate lobe, mild coarse liver texture, nr hepatic flow 2 nd : hepatomegaly, prominent caudate, no focal mass, acute thrombosis of portal and splenic vein. Huge splenomegaly and moderate abdominal Ascitis. Last PT: 24.7 INR: 3
  10. 10. Final diagnosis: Portal vein/splenic vein thrombosis due to hypercoagulable state from ?essential throbocythemia potentate by oral contraceptive use.
  11. 11. OTHER CASE REPORTS FROM INTERNET & LITERETURE:
  12. 12. Case 2 52 year old man with a history of PNH originally diagnosed in 1978 (at age 28) when he presented with dark urine and was noted to have moderate hemolytic anemia Treated initially at that time with prednisone and blood transfusions. Had a relatively mild course with infrequent episodes of hematuria and very few blood transfusions Did not see a hematologist for over 20 years
  13. 13. Case Presentation Presented in April 2002 with abdominal pain and distension and was noted to be jaundiced Hepatitis workup was negative but an MRI of the abdomen revealed hepatic vein thrombosis (Budd-Chiari syndrome) Underwent a transjugular liver biopsy on 4/23/02 which revealed extensive fibrosis, no cirrhosis, sinusoidal dilatation and central vein fibrosis consistent with Budd-Chiari syndrome Despite anticoagulation and numerous paracenteses, refractory ascites was present and he underwent a TIPS procedure in 7/02
  14. 14. Case Presentation Patient subsequently developed hepatic encephalopathy, requiring multiple hospital admissions over 2 months following TIPS placement Ultrasounds confirmed patency of shunt Referred to Dr Bessler in 9/02. Evaluation revealed a jaundiced, mildly encephalopathic man with normal abdomen CBC revealed Hgb 11.9 with MCV 97.2, plt cnt 90,000, ANC 2,700, INR 1.7, creatinine 0.8, and bilirubin 6
  15. 15. Case Presentation <ul><li>Flow Cytometry: </li></ul><ul><ul><li>erthrocytes: 17% CD59 deficient </li></ul></ul><ul><ul><li>granulocytes: 99.8% CD59/CD24 deficient </li></ul></ul><ul><ul><li>monocytes: 82% CD59 deficient </li></ul></ul><ul><ul><li>lymphocytes: 24% CD59 deficient </li></ul></ul><ul><li>Anticoagulation increased, folic acid started, and referred for consideration of hematopoietic stem cell transplantation </li></ul>
  16. 16. Case: 3
  17. 20. Case:4
  18. 21. Case: 5,6
  19. 22. Case: 1 More cases
  20. 24. Vascular diseases of the liver; Budd-chiari/portal vein thrombosis& other veno-occlusive disorders: Conditions in which radiologists & interventional radiologists can contribute much. Prepared by: Dr.Mohammad Shaikhani. [email_address]
  21. 25. Definition <ul><li>Heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the level of the hepatic venules, the large hepatic veins, the IVC, or the right atrium </li></ul>
  22. 26. Pathogenesis <ul><li>Obstruction of the hepatic venous outflow tract </li></ul><ul><li>Increased hepatic sinusoidal pressure /portal hypertension. </li></ul><ul><li>Portal venous perfusion of the liver is decreased, which may result in portal venous thrombosis. </li></ul><ul><li>The ensuing venous stasis and congestion lead to hypoxic damage to adjacent hepatic parenchymal cells& the ischemic injury to the sinusoidal lining cells results in the release of free radicals& oxidative injury to the hepatocytes ensues with hepatocyte necrosis in the centrilobular regions, progressive centrilobular fibrosis, nodular regenerative hyperplasia, ultimately, cirrhosis </li></ul><ul><li>If the hepatic sinusoidal pressure is reduced by the creation of a portosystemic shunt or by the development of a portal venous collateral system, liver function improves. </li></ul>
  23. 28. BCS:Blood disorders <ul><li>Major causes of vascular liver diseases. </li></ul><ul><li>Atypical myeloproliferative diseases most commonly implicated. </li></ul><ul><li>Frequent combination of several causes. </li></ul><ul><li>Permanent anticoagulation is generally recommended once prophylaxis for portal hypertensive bleeding has been instituted. </li></ul>
  24. 29. Clinical Manifestations <ul><li>Depends on the extent &rapidity of the hepatic-vein occlusion & whether a venous collateral circulation has developed to decompress the sinusoids. </li></ul><ul><li>Four Types </li></ul><ul><li>Fulminant </li></ul><ul><li>Acute </li></ul><ul><li>Subacute </li></ul><ul><li>Chronic </li></ul>
  25. 30. Clinical Presentation <ul><li>Fulminant </li></ul><ul><li>hepatic encephalopathy within eight weeks of development of jaundice; uncommon </li></ul><ul><li>Acute </li></ul><ul><li>symptoms of short duration, intractable ascites, hepatic necrosis with the formation of venous collaterals; usually thrombosis of all major hepatic veins </li></ul>
  26. 31. Clinical Presentation <ul><li>Subacute </li></ul><ul><li>most common; more insidious onset </li></ul><ul><li>ascites& hepatic necrosis may be minimal; 1/3 of patients with thrombosis of all major hepatic veins </li></ul><ul><li>Chronic </li></ul><ul><li>manifest as cirrhosis </li></ul>
  27. 32. Clinical Presentation <ul><li>Abdominal pain, hepatomegaly and ascites are present in almost all patients with Budd-Chiari syndrome </li></ul><ul><li>Nausea, vomiting and mild jaundice is more frequent in fulminant and acute forms </li></ul><ul><li>Splenomegaly and esophagogastric varices may be present in the chronic form </li></ul>
  28. 33. Diagnosis <ul><li>Serum AST / ALT may be >*5 ULN in the fulminant &acute forms, whereas increases are smaller in the subacute form. </li></ul><ul><li>Serum alkaline phosphatase /bilirubin also increase to a varying extent, along with a decrease in serum albumin. </li></ul><ul><li>The serum–ascitic fluid albumin gradient is high, with the total protein level in the ascitic fluid usually > 2.5 g per deciliter, similar to ascites of cardiac disease. </li></ul><ul><li>Doppler ultrasonography of the liver, with a sensitivity/pecificity of 85 percent or more, is the technique of choice for initial investigation when suspected. </li></ul><ul><li>Necrotic areas of the liver are better seen, on contrast-enhanced CT, which may be recommended to delineate the venous anatomy & the configuration of the liver when a TIPS is being considered. </li></ul><ul><li>MRI with VIBE sequence is better for visualizing the whole length of the inferior vena cava & may permit differentiation of the acute form from the subacute & chronic forms. </li></ul><ul><li>In some patients, echoc may be needed to rule out tricuspid regurgitation, constrictive pericarditis, or a right atrial myxoma. </li></ul>
  29. 34. Diagnosis <ul><li>Confirmed by a “spiderweb” pattern on hepatic venography& the confirmation is necessary, even with a negative result on ultrasonographic exam, when there is a strong clinical suspicion. </li></ul><ul><li>The inferior vena cava may have a thrombus or may be compressed by an enlarged caudate lobe. </li></ul><ul><li>Measurement of the portacaval venous pressure gradient (the portal-vein pressure minus the infrahepatic vena caval pressure) may be useful in determining the likelihood that portacaval shunting will be successful. </li></ul><ul><li>If possible, a transjugular liver biopsy of both the right and left lobes should be carried out at the time of the angiographic investigation to confirm the diagnosis and to help guide therapy, though the appropriateness of biopsy has not yet been established. </li></ul>
  30. 35. Diagnosis
  31. 39. Management <ul><li>Medical </li></ul><ul><li>Sodium restriction </li></ul><ul><li>Furosemide </li></ul><ul><li>Spironolactone </li></ul><ul><li>Paracentesis </li></ul><ul><li>Heparin /Warfarin INR 2.0-2.5 </li></ul>
  32. 40. Management
  33. 42. Management
  34. 43. Management
  35. 46. Etiologies of PVT Cirrhosis is most common - 10-30% 10-50% 40-60% IBD Pregnancy Blunt Trauma OCPs Portocaval Shunt Peptic Ulcer Dz Elevated Homocysteine Protein C/S deficiency Umbilical Vein Catheters Appendicitis Anti-cardiolipin Anti-thrombin III Colectomy Diverticulitis APL syndrome Prothrombin Mutation 20210G/A Splenectomy Pancreatitis Malignancy FVL Liver Transplant Sepsis Acquired Inherited Surgical Inflammatory Thrombophilia Local Factors
  36. 47. Etiologies of PVT <ul><li>PVT without liver disease is rare </li></ul><ul><ul><li>Cirrhotics with PVT </li></ul></ul><ul><ul><ul><li>11%-27% </li></ul></ul></ul><ul><ul><li>General Population </li></ul></ul><ul><ul><ul><li>unknown </li></ul></ul></ul><ul><li>Non-cirrhotic PVT </li></ul><ul><ul><li>Local Factors </li></ul></ul><ul><ul><ul><li>10-50% </li></ul></ul></ul><ul><ul><li>Thrombophilia </li></ul></ul><ul><ul><ul><li>40-60% </li></ul></ul></ul>IBD Blunt Trauma Pregnancy PUD OCPs Portocaval Shunt Appendicitis Elevated Homocysteine Protein C/S deficiency Umbilical Vein Catheters Diverticulitis Anti-cardiolipin Anti-thrombin III Colectomy Pancreatitis APL syndrome Prothrombin Mutation Splenectomy Sepsis Malignancy FVL Liver Transplant Cirrhosis Acquired Inherited Surgical Inflammatory Thrombophilia Local Factors
  37. 48. Consequences of PVT <ul><li>Pain, nausea and vomiting, anorexia </li></ul><ul><li>Bowel Ischemia </li></ul><ul><ul><li>From venous congestion </li></ul></ul><ul><li>Hypersplenism traits: thrombocytopenia </li></ul><ul><li>Bleeding Esophageal Varices </li></ul><ul><ul><li>Manifests in 30% of cases </li></ul></ul><ul><ul><li>Lower morbidity and less rebleeding in non-cirrhotics* </li></ul></ul>
  38. 49. Consequences of PVT <ul><li>Pain, nausea and vomiting, anorexia </li></ul><ul><li>Bowel Ischemia </li></ul><ul><ul><li>From venous congestion </li></ul></ul><ul><li>Hypersplenism traits: thrombocytopenia </li></ul><ul><li>Bleeding Esophageal Varices – 30% of cases </li></ul><ul><ul><li>Lower frequency of rebleeding in non-cirrhotics (Condat) </li></ul></ul><ul><ul><li>Vleggaar (1998) - Endoscopic VBL highly successful </li></ul></ul><ul><ul><ul><li>95% 5 year survival rate </li></ul></ul></ul><ul><ul><ul><li>No mortality related to recurrent bleeding </li></ul></ul></ul>
  39. 50. Treatment of PVT and the Prothrombin mutation <ul><li>Unclear and nonspecific in literature </li></ul><ul><li>In cirrhosis - traditional portal HTN treatment alone </li></ul><ul><li>Debate of anticoagulation use in non-cirrhotic PVT </li></ul><ul><li>- Condat and Sheen – 78-90% show resolution or </li></ul><ul><li>recanalization of the thrombus </li></ul><ul><li> </li></ul><ul><li>Vague Recommendations with Anticoagulation </li></ul><ul><li>Does the Prothrombin mutation warrant prophylactic anticoagulation? </li></ul>
  40. 51. Other References: <ul><li>www.uptodate.com </li></ul><ul><li>K.V. Narayanan Menon, M.D., Vijay Shah, M.D., and Patrick S. Kamath, M.D. The Budd-Chiari Syndrome. N Engl J Med 2004;350:578-85. </li></ul><ul><li>Cheryl J. Davis, MD, internet case presentation & review: November 27, 2007. </li></ul><ul><li>Portal Vein Thrombosis, An Unexpected Finding in a 28 year old male with Abdominal Pain, internet case presentation & review; Jason Ferguson, DO CPT, MC, USA; Tripler Army Medical Center; Honolulu, Hawaii </li></ul>

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